Virchows Archiv A

, Volume 406, Issue 3, pp 373–380

Multiple paraganglioneuromas


  • Kenji Kawaguchi
    • Department of PathologyTokyo Metropolitan Komagome Hospital
  • Touichiro Takizawa
    • Department of PathologyTokyo Metropolitan Komagome Hospital
  • Morio Koike
    • Department of PathologyTokyo Metropolitan Komagome Hospital
  • Ikuo Tabata
    • Department of MedicineTokyo Metropolitan Komagome Hospital
  • Narihide Goseki
    • Department of SurgeryTokyo Metropolitan Komagome Hospital
Case Report

DOI: 10.1007/BF00704306

Cite this article as:
Kawaguchi, K., Takizawa, T., Koike, M. et al. Vichows Archiv A Pathol Anat (1985) 406: 373. doi:10.1007/BF00704306


We report multiple paraganglioneuromas which occurred in a 40-year-old-man. Thirty-two tumours with similar histological appearance have been reported previously and most of them showed a striking predilection to occur in the second portion of the duodenum. In this case, three masses were detected; one was located in the periampullary region of duodenum with a polypoid appearance, the others were well defined masses in peri-pancreatic tissue adjacent to large vessels. Histology revealed two cellular components, epithelioid cells with NSE immunoreactivity and S-100 protein containing spindle-shaped cells. Moreover, on electron microscopical examination, three different epithelioid cell types were seen. Type I was a figure differentiating to ganglion cells, type II to paraganglion cells, type III was a hybrid form of ganglion and paraganglion cells. Paraganglioneuroma revealed the histopathology of ganglioneuroma, paraganglioma and also a mixed appearance. In this respect the tumour may be considered to originate in undifferentiated neural crest cells and develop organoid differentiation.

Key words

ParaganglioneuromaParagangliomaGanglioneuromaGangliocytic paragangliomaHybrid cell

Copyright information

© Springer-Verlag 1985